Greetings from below the aortic arch! The paradigm of cardiac paraganglioma.

A 42-yr-old female, mother of five children, was referred to this institution for further evaluation of recurrent episodes of severe arterial hypertension. Blood pressures taken on several occasions during the past 3 yr by her general practitioner had documented recurrent hypertensive crises with systolic/diastolic measurements of up to 300/145 mm Hg. These episodes were frequently accompanied by headache, palpitations, sweating, nausea, and pallor followed by weakness. Her past medical history was remarkable only for transient hepatitis B following blood transfusion in 1970 and Cesarian section in 1986. Family history was negative for multiple endocrine neoplasia (MEN) type 2A and 2B syndromes, von Recklinghausen’s neurofibromatosis, and von Hippel-Lindau’s disease. Her medications included intermittent usage of furosemide (40 mg/day) for leg edema and L-thyroxine (200 pg/day) for a history of hypothyroidism. Diagnostic tests before referral had revealed an elevated 24-h urinary excretion of vanillylmandelic acid at 27 mg/24 h (0.3-7.7 mg/24 h) and normal 24-h urinary excretion of catecholamines. In addition, on computed tomography of the abdomen, a right adrenal mass had been detected. Physical examination revealed moderate obesity (170 cm, 83 kg) and marked hypertension (180/115 to 230/120 mm Hg) but was otherwise normal. No heart murmurs were heard, and fundoscopic and skin examination were unremarkable. Twentyfour hour blood pressure monitoring demonstrated a systolic mean of 170 mm Hg without diurnal rhythm. Serum electrolytes were normal except for mildly elevated corrected calcium levels of 2.6-2.8 mmol/L (2.0-2.5 mmol/L) and a low potassium level of 3.3 mmol/L (3.5-5.5 mmol/L). All other routine blood tests were within normal limits. Total 24-h urinary catecholamine measurements revealed markedly elevated concentrations of norepinephrine at 912 pg/24 h (cl00 pg/24 h), metanephrines at 4.5 mg/24 h (<1.3 mg/24 h), and vanillylmandelic acid at 18 mg/24 h (<8 mg/24 h), whereas epinephrine and dopamine concentrations were normal. Thyroid function tests revealed complete suppression of basal TSH at CO.03 mU/L (0.4-4.0 mU/